关于血小板减少症的二三事.pptx

关于血小板减少的二三事Thrombocytopenia生理学小知识1 造血干细胞→巨核系祖细胞→原始巨核细胞→巨幼核细胞→成熟巨核细胞megakaryocyte→血小板 （8-10d）2 约2/3在外周循环血液中，1/3在脾脏肝脏3 寿命 7-14d4 最小血细胞，无细胞核，双面微凸圆盘状，D 2-3μm5 黏附、释放、聚集、收缩、吸附等生理特性6 →维持血管内皮的完整性 →凝血止血想到血小板Asymptomatic, thrombocytopenia first detected on a routine complete blood count.Clinical Characteristics of Bleeding DisordersFeaturePlatelet/vascular defectCoagulation defectSiteSkin, mucous membranes*Deep in soft tissues (muscle, joints)LesionsPetechiae 瘀点, Ecchymoses 瘀斑*Hemarthroses 关节积血,Hematomas 血肿BleedingAfter minor cuts: yesAfter surgery: immediate, mildAfter minor cuts: unusualAfter surgery: delayed, severeClinical manifestationsSkin: petechiae 瘀点, superficial ecchymoses瘀斑Mucous membranes: epistaxis 鼻衄, gingival bleeding牙龈出血, large bullous hemorrhages on the buccal mucosa 颊粘膜大血疱 Menorrhagia月经过多, metrorrhagia子宫不规则出血Most common cause of death→ preceding trauma→Bleeding into the central nervous systemPurpura 紫癜Nonblanching purple/red lesions due to extravasation of RBCs into dermisNonpalpable (macular; ≤3 mm in diameter= petechiae; ＞3 mm = ecchymoses) platelet disorder: thrombocytopenia, defect in platelet function thromboemboli: DIC,TTP, cholesterol or fat emboli trauma or vascular fragility: amyloidosis, Ehlers-Danlos, scurvyPalpable (papular) vasculitis: leukocytoclastic, HSP, PAN, RMSF infectious emboli: meningococcemia, bacterial endocarditisRisk of Plt↓Thrombocytopenia→Plt ＜100,000/μLThrombocytopenia and Risk of BleedingPlatelet count (cells/μL)Risk50,000-100,000Risk with major trauma; can proceed with general surgery20,000-50,000Risk with minor trauma or surgery＜20,000Risk of spontaneous bleeding (less so with ITP)＜10,000Risk of severe, life-threatening bleedingEtiologiesExclude pseudothrombocytopeniaTrue thrombocytopenia ↓production ↑destruction Abnormal distribution or pooling UnknownPseudothrombocytopeniaPlatelet clumping in EDTA0.1% have EDTA- dependent agglutinins 凝集素a naturally occurring platelet autoantibody directed against a normally concealed epitop